Subject(s)
Multiple Myeloma/secondary , Plasma Cells/pathology , Pleural Effusion, Malignant/pathology , ADP-ribosyl Cyclase 1/analysis , Aged , Antigens, CD/analysis , Antigens, Neoplasm/analysis , Chromosome Aberrations , Combined Modality Therapy , Disease Progression , Fatal Outcome , Humans , Immunophenotyping , In Situ Hybridization, Fluorescence , Male , Membrane Glycoproteins/analysis , Multiple Myeloma/pathology , Multiple Myeloma/therapy , Pleural Effusion, Malignant/genetics , Pleural Effusion, Malignant/therapy , Polyploidy , RecurrenceABSTRACT
The autoimmune GFAP astrocytopathy has been associated with meningoencephalomyelitis that usually responds to glucocorticoids. We report a 20-year-old man that developed an acute and severe meningoencephalomyelitis with remarkable CNS hyperexcitability and oculogyric crises. CSF analysis showed hypoglycorrhachia, pleocytosis, elevated ADA, and CSF-immunofluorescence characteristic of autoimmune GFAP astrocytopathy. MRI showed lesions at thalamus, corpus-callosum, dorsal pons and dentate nucleus with associated myelitis. Immunotherapy led to a full recovery, although MRI activity was observed at follow-up. CNS hyperexcitability, typically seen in other immune-mediated syndromes, represents a novel presenting form to be included as part of the clinical spectrum of this entity.